儿童交替性偏瘫(附3例报告)

目的探讨儿童交替性偏瘫（AHC）的临床特点和治疗方法。方法对3例AHC患儿的临床资料进行分析。结果3例患儿的临床特征为生后18个月内起病．反复发作、时间不等的交替性偏瘫，伴有眼位异常，肌张力异常，舞蹈样徐动动作，植物神经机能紊乱和智力障碍；睡眠可缓解。结论本病的主要特征为生后18个月内起病的发作性交替性偏瘫，伴有锥体外系症状及智能障碍，Flunarizine治疗有效。     

儿童交替性偏瘫六例分析

目的探讨儿童交替性偏瘫（ＡＨＣ）的临床特点及治疗方法。方法对６例ＡＨＣ患儿的临床资料进行分析。结果６例患儿的临床特征为出生后１８个月起内病，频繁发作，持续数分钟至数小时；短暂的眼球震颤，肌张力异常，舞蹈徐动样动作，植物神经机能紊乱和认知机能减退；睡眠可缓解无力及锥体外系症状。应用氟桂嗪治疗后，１例患儿发作完全停止，其余５例患儿均显示发作频率和持续时间降低。结论本病的主要特征为１８个月内起病的发作性交替性偏瘫，伴锥体外系症状及智力障碍，氟桂嗪治疗本病有效     

单光子发射计算机断层扫描对致痫灶定位价值探讨

对１０５例癫痫患者在发作间期进行了单光子发射计算机断层扫描（ＳＰＥＣＴ）检查，并对其中２０例在发作期也进行了扫描。该２０例中，发作期局部脑血流均较发作间期有不同程度提高，将两组图像经减影处理后得出一组减影图像，该减影图像可显示出血流改变区即致痫灶的具体大小及形态，以此结果指导手术。１０例发作完全控制；６例发作减少７５％以上；３例减少５０％；１例无改善，总有效率达９５％。术后半年对其中５例进行了ＳＰＥＣＴ复查，并与术前发作间期进行减影处理，发现除皮质切除部位外，脑血流灌注均有不同程度改善。     

三例儿童交替性偏瘫的回顾性分析

目的探讨儿童交替性偏瘫病因、临床特点、治疗效果。方法回顾性分析3例儿童交替性偏瘫患者的病因、临床表现及疗效。结果本组3例患者起病年龄均小于18个月,反复发作的交替性偏瘫；进行性的智能障碍,其中1例伴有短暂眼球震颤及眼球活动障碍,1例伴有张力障碍性姿势异常；睡眠可缓解无力及锥体外系症状,应用氟桂嗪治疗后,2例患者发作频率及持续时间降低,1例无效。结论本病病因不明,多为散发,临床表现为18月内起病的发作性交替性偏瘫,辅助检查无特征性改变,氟桂利嗪治疗部分有效。     

儿童额叶癫痫的临床特征以及切除性手术预后研究——儿童癫痫中心的经验

目的 分析儿童额叶癫痫的临床特征、手术预后以及两者的相关性。方法 回顾性分析2017年1月—2019年12月复旦大学附属儿科医院三级癫痫中心收治并进行额叶致痫灶切除手术的额叶癫痫患儿18例,收集临床、头颅影像学、脑电图、智力发育以及病理等资料,术后随访至少2年以上并记录癫痫发作情况,利用单因素方法分析手术预后和临床表型之间的相关性。结果 18例患儿中男11例、女7例,平均手术年龄（6.8±2.73）岁,年龄范围21月龄～11岁,病程1个月～9年。17例表现为局灶性发作、1例表现为全面性癫痫性痉挛发作。发作间期额区放电11例、额区外多脑区放电7例,发作期额区起始9例、额区外起始9例。核磁共振成像阳性改变14例、阴性4例。病因分别为局部皮层发育不良14例、发育性肿瘤2例、结节性硬化和胶质增生各1例。致痫病灶直接切除11例、立体定向脑电图深部电极植入后致痫灶切除7例。根据Engel分级,术后2年Ⅰ级14例（77.8%）、Ⅱ级1例（5.6%）、Ⅲ级2例（11.0%）、Ⅳ级1例（5.6%）。术后预后在性别、起病年龄、病程、致痫灶侧别、核磁共振成像阳性发现、发作间期放电和发作期放电起始、病因、智力...     

SPECT显像与美解眠诱发试验对癫癎病灶定位诊断价值的探讨

目的探讨美解眠激发癫癎脑血流灌注显像对癫癎致癎灶的定位诊断价值,为立体定向放射治疗提供定位依据.方法32例药物治疗失败并准备r-刀手术治疗的癫癎患者,发作间期行SPECT检测;应用美解眠诱发,观察亚临床发作期SPECT的变化.结果32例癫癎患者,发作间期SPECT呈低灌注者26例,阳性率81.3%,而MR、x-CT阳性率分别为56.2%和40.6%.SPECT显示病灶部位42个,于颞叶位病灶最多,占47.6%,其次为额叶、枕叶、顶叶等.发作间期SPECT 26例低灌注者及其余6例正常灌注中的3例,用美解眠诱发后的亚临床发作期SPECT均出现脑血流增多,呈(相对)高灌注,其中6例高灌注患者病灶范围较低灌注区域减少.这29例患者r-刀手术治疗有效率达86.2%.结论发作间期SPECT的脑血流低灌注结合美解眠诱发的亚临床发作期的SPECT脑血流灌注增高,对癫癎病灶定位诊断具有重要的临床意义.     

伴中央中颞区棘波的儿童良性癫(癎)患者治疗前后异常SPECT灌注灶的变化

目的:观察伴中央中颞区棘波的儿童良性癫(癎)(BECT)长期控制中发作间期单光子发射断层扫描(SPECT)异常灌注灶恢复情况和V-EE(的变化.方法:观察25例BECT患儿经抗癫(癎)药治疗前后发作间期SPECT显像与长程EEG及影像学的变化.结果:控制发作时间平均30个月,用药前后SPECT两次比较:用药后SPECT...     

Menkes病继发癫痫：五例报告并文献复习

目的 研究以癫痫发作为首发表现的Menkes病临床特点,提高对继发性癫痫病因诊断的认识。方法 回顾分析5例Menkes病患儿的临床、实验室和影像学特点。结果 5例患儿均为男婴,其中2例为兄弟,发病年龄1-5个月,表现为强直发作（3例）或局灶性阵挛发作（2例）,后者发作频繁且呈进行性加重。其中,4例脑电图背景波异常、3例发作间期以枕区为主的局灶性或多灶性样放电、2例发作期性放电;5例患儿均伴明显发育迟滞,皮肤白皙、毛发卷曲;4例血清铜和铜蓝蛋白降低明确诊断为Menkes病、1例因兄长已先证诊断结合自身典型表现临床诊断为Menkes病;3例MRI均可见广泛脑萎缩和对称性脑软化灶、1例MRA可见颅内大动脉扭曲和末端变细。分别接受苯巴比妥（1例）、托吡酯（2例）或托吡酯＋左乙拉西坦＋氯硝安定联合治疗（1例）。1例住院24 h死于呼吸衰竭,4例出院后随访1~9个月,3例死亡,1例随访4个月后转为频繁痉挛发作伴发育迟缓,发作间期脑电图呈现高峰失律。结论 Menkes病继发癫痫多于婴儿期发病,早期以强直发作或局灶阵挛发作为主要表现伴发作间期样放电,抗癫痫药物难以控制发作,患儿预后不良。     

Menkes病继发癫:五例报告并文献复习

目的研究以癫发作为首发表现的Menkes病临床特点,提高对继发性癫病因诊断的认识。方法回顾分析5例Menkes病患儿的临床、实验室和影像学特点。结果 5例患儿均为男婴,其中2例为兄弟,发病年龄1~5个月,表现为强直发作(3例)或局灶性阵挛发作(2例),后者发作频繁且呈进行性加重。其中,4例脑电图背景波异常、3例发作间期以枕区为主的局灶性或多灶性样放电、2例发作期性放电;5例患儿均伴明显发育迟滞,皮肤白皙、毛发卷曲;4例血清铜和铜蓝蛋白降低明确诊断为Menkes病、1例因兄长已先证诊断结合自身典型表现临床诊断为Menkes病;3例MRI均可见广泛脑萎缩和对称性脑软化灶、1例MRA可见颅内大动脉扭曲和末端变细。分别接受苯巴比妥(1例)、托吡酯(2例)或托吡酯+左乙拉西坦+氯硝安定联合治疗(1例)。1例住院24 h死于呼吸衰竭,4例出院后随访1~9个月,3例死亡,1例随访4个月后转为频繁痉挛发作伴发育迟缓,发作间期脑电图呈现高峰失律。结论 Menkes病继发癫多于婴儿期发病,早期以强直发作或局灶阵挛发作为主要表现伴发作间期样放电,抗癫药物难以控制发作,患儿预后不良。     

探讨儿童部分性癫痫发作发作间期脑电图及与影像学的关系

目的探讨儿童部分性癫痫发作发作间期EEG及与影像学的关系。方法对67例部分性发作的癫痫患儿行长程录像脑电监测(VEEG)及影像学检查,分析发作间期EEG及与影像学的关系。结果与发作间期EEG正常的患儿比较,EEG异常患儿影像学正常率显著降低,影像学异常率显著增高(χ2=5.154,P=0.023)。发作间期异常放电51例患者中,42例(82.4%)间期放电与发作期部位一致,其中20例(47.6%)影像学检查正常,22例(52.4%)影像学检查异常,两者相比差异无统计学意义(P=0.726)。结论发作间期EEG异常部分性癫痫患儿,影像异常率高,大多数患儿发作间期放电与发作期起始放电部位一致。     

Single photon emission computed tomography findings in a case of alternating hemiplegia of childhood in relation to migraine

Single photon emission computed tomography (SPECT) was performed 3 times during attacks and performed 4 times during postictal periods on a case of alternating hemiplegia of childhood. Hyperperfusion of the corresponding hemisphere to hemiparesis was suggested by asymmetric increase RI uptake during the ictal scans, whereas interictal scans showed symmetric topography of cerebral blood flow. Manifestations except hemiplegia included loss of consciousness and vomiting. These manifestations and ictal SPECT findings are the same evidence as hemiplegic migraine. It suggests that these two disorders have a similar pathophysiology. There are two interesting findings regarding this patient. The first finding is magnetic resonance imaging showed progressive cerebellar atrophy. Second finding is interictal SPECT showed a progressive decrease of cerebral perfusion, especially in cerebellar hemispheres. These two findings suggest alternating hemiplegia of childhood may be a chronic progressive disorder.     

Benign nocturnal alternating hemiplegia of childhood: A new case with unusual findings

It has been described a neuro developmental disorder labelled “Benign nocturnal alternating hemiplegia of childhood” (BNAHC) characterized by recurrent attacks of nocturnal hemiplegia without progression to neurological or intellectual impairment. We report a female patient who at 11 months revealed a motionless left arm, unusual crying without impairment of consciousness and obvious precipitating factors. The attacks occur during sleep in the early morning with lack of ictal and interictal electroencephalographic abnormalities, progressive neurological deficit, and cognitive impairment. Unlike previous reports of BNAHC our patient come from a family with a history of both migraine, hemiplegic migraine, and sleep disorders. Our study remarks on the typical features described in previous studies and stresses the uncommon aspects that could help to identify the disorder which is likely to have been underestimated. Despite some clinical similarities between BNAHC and familiar hemiplegic migraine and alternating hemiplegia of childhood, the genetic analyses of our patient did not reveal genetic mutations found in both disorders.     

Alpha[11C] methyl-L-typtophan positron emission tomography in patients with alternating hemiplegia of childhood

Based on previous reports suggesting a role of the neurotransmitter serotonin in the pathomechanism of alternating hemiplegia of childhood and speculation that it may be a migraine variant, we measured brain serotonin synthesis in children with alternating hemiplegia of childhood. Clinical and neurodevelopmental data, as well as standard uptake values in 25 brain regions and whole-brain serotonin synthesis capacity (unidirectional uptake rate constant or K-complex), were assessed in six patients with alternating hemiplegia of childhood (three girls and three boys; mean age = 7 6/12 years) using alpha[11C]methyl-L-tryptophan positron emission tomography (PET). The PET studies were performed interictally in three patients, during the ictal state in two patients, and postictally in one patient. The PET data were compared to those obtained interictally from six age-matched patients with focal epilepsy (two girls and four boys; mean age = 7 8/12 years) and six non-age-matched apparently normal siblings of autistic children (two girls and four boys; mean age = 9 11/12 years). Patients with alternating hemiplegia of childhood studied in the ictal or postictal state showed increased serotonin synthesis capacity in the frontoparietal cortex, lateral and medial temporal structures, striatum, and thalamus when compared to controls, and subjects with alternating hemiplegia of childhood studied interictally. The involvement of these brain regions was consistent with the semiology of the hemiplegic attacks. In patients with interictal studies and in the controls, the PET scans revealed similar and bilaterally symmetric regional patterns of serotonin synthesis capacity. Increased whole-brain serotonin synthesis capacity (reported in migraine subjects without aura) was not found in the alternating hemiplegia of childhood group. There was no correlation between the neurodevelopmental scores and regional standard uptake values; however, patients with a larger estimated lifetime attack number showed greater delay in communication (P = .005) and daily living skills (P = .042). These studies suggest increased regional serotonergic activity associated with attacks in alternating hemiplegia of childhood. Furthermore, the attack number may have an effect on neurodevelopmental delay, thus supporting the notion that alternating hemiplegia of childhood may be a progressive disorder.     

Focal brain dysfunction in a 41-year old man with familial alternating hemiplegia

The acute pathophysiologic changes during hemiplegic spells and the long-term outcome of alternating hemiplegia remain obscure. In a 41-year-old male with familial alternating hemiplegia we found an increase in right frontal cerebral blood flow 3 h into a 5-h left hemiplegic episode. A repeat high-resolution brain SPECT study performed 26 h after the resolution of the left hemiplegia revealed normalization of the frontal blood flow accompanied by hyperperfusion in the right parietal lobe. An interictal SPECT scan several weeks later showed no asymmetries. Head CT and MRI scans were negative. Neuropsychologic assessment and neurologic examination revealed evidence of a diffuse disorder which predominantly involved the right hemisphere. To our knowledge, there are no previous correlative studies of serial highresolution brain SPECT with MRI, or of detailed neuropsychologic assessment, in adult patients with such an advanced course of alternating hemiplegia of childhood.     

123I-iodoamphetamine SPECT brain imaging in alternating hemiplegia

Alternating hemiplegia of childhood is an unusual disorder characterized by early onset (occurring before 18 months of age); repeated attacks of hemiplegia involving both sides of the body; other paroxysmal phenomena, such as tonic stiffening, dystonic posturing, choreoathetoid movements, ocular motor abnormalities, and autonomic disturbances, in association with bouts of hemiplegia or occurring independently; and evidence of mental or neurologic deficits. A girl was examined because of left hemiplegia at the age of 16 months. The patient had begun exhibiting episodes of alternating hemiplegia at approximately 4 months of age. They consisted of tonic stiffening and dystonia of the right or left extremities, lasting from 30 min to several hours and followed by residual hemiparesis. They were invariably accompanied by ocular motor abnormalities. Magnetic resonance imaging, computed tomography, and angiography all were normal. Single proton emission computed tomography brain images during an acute episode of right hemiplegia demonstrated hypoperfusion of the left cerebral hemisphere. Following improvement of the hemiplegia, the patient was re-evaluated. The uptake of the radiotracer in the left hemisphere was increased. The scan did not demonstrate significant asymmetry in cerebral perfusion.     

Ictal 99mTc-HMPAO SPECT in alternating hemiplegia

99mTc-hexamethylpropylenamine oxime (99mTc-HMPAO) single-photon emission computed tomography (SPECT) was performed in a patient with alternating hemiplegia during 2 episodes of the disease. The regional cerebral blood flow patterns correlated with the clinical manifestations during both episodes. Hyperperfusion of the contralateral hemisphere was suggested by asymmetric 99mTc-HMPAO uptake, whereas symmetric 123I-N-isopropyl-p-iodoamphetamine uptake was detected during the interictal period. The results suggested that alternating hemiplegia in infants represents an atypical manifestation of epilepsy, despite the lack of paroxysmal electroencephalographic abnormalities during the episodes. 99mTc-HMPAO SPECT appears to be a useful method for detecting transient regional cerebral blood flow alterations during paroxysmal events because the tracer is rapidly available for emergencies and retains a fixed distribution for 5-8 hours, sufficient time to allow for SPECT acquisition.     

Epileptic seizures difficult to differentiate from alternating hemiplegia in infants: a case report

A child with epileptic seizures had a clinical course similar to that of alternating hemiplegia of infancy (AHI). Tonic hemiplegia began at 2 months of age, and atonic alternating hemiplegia and tetraplegic attacks began at 2 years of age. Clinical findings were paroxysmal ocular movement abnormalities, choreoathetotic involuntary movements, and severe developmental retardation. An interictal EEG at 6 years of age showed multiple independent spike discharges. An ictal EEG showed diffuse irregular spike-wave and slow wave bursts following focal spikes. The present case suggests that a long-term extensive follow-up is necessary to differentiate epileptic seizures from AHI.     

Alternating hemiplegia of childhood: Studies of regional cerebral blood flow using 99mTc-hexamethylpropylene amine oxime single-photon emission computed tomography

Alternating hemiplegia of childhood is a rare disorder of unknown cause associated with progressive neurological deterioration. We report the results of regional cerebral blood flow studies using ^99mTc-hexamethylpropylene amine oxime single-photon emission computed tomography in 3 patients. These studies were performed during the hemiplegic attacks (n = 6) and during the symptom-free periods (n = 2). Six single-photon emission computed tomographic studies performed during hemiplegic attacks consistently showed relative hyperperfusion of the contralateral cerebral hemisphere. Two single-photon emission computed tomographic studies performed during the asymptomatic phase showed normal and symmetrical cerebral perfusion. This is the first definite demonstration of unilateral increase of cerebral blood flow in alternating hemiplegia. These findings support the possibility of a relationship between the cause of alternating hemiplegia and migraine.